General Information About Childhood Pancreatic Cancer

Malignant pancreatic tumors are rare in children and adolescents, with an incidence of 0.46 cases per 1 million individuals younger than 30 years.[1,2,3,4]

The primary pancreatic tumors of childhood can be classified into the following four categories:

• Solid pseudopapillary tumor of the pancreas.
• Pancreatoblastoma.
• Islet cell tumor.
• Pancreatic carcinoma.

References:

1. Chung EM, Travis MD, Conran RM: Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 26 (4): 1211-38, 2006 Jul-Aug.
2. Perez EA, Gutierrez JC, Koniaris LG, et al.: Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients. J Pediatr Surg 44 (1): 197-203, 2009.
3. Dall'igna P, Cecchetto G, Bisogno G, et al.: Pancreatic tumors in children and adolescents: the Italian TREP project experience. Pediatr Blood Cancer 54 (5): 675-80, 2010.
4. Brecht IB, Schneider DT, Klöppel G, et al.: Malignant pancreatic tumors in children and young adults: evaluation of 228 patients identified through the Surveillance, Epidemiology, and End Result (SEER) database. Klin Padiatr 223 (6): 341-5, 2011.

Special Considerations for the Treatment of Children With Cancer

Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[1] Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the following pediatric specialists and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life:

• Primary care physicians.
• Pediatric surgeons.
• Pathologists.
• Pediatric radiation oncologists.
• Pediatric medical oncologists and hematologists.
• Ophthalmologists.
• Rehabilitation specialists.
• Pediatric oncology nurses.
• Social workers.
• Child-life professionals.
• Psychologists.
• Nutritionists.

For specific information about supportive care for children and adolescents with cancer, see the summaries on Supportive and Palliative Care.

The American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of children and adolescents with cancer.[2] At these centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate is offered to most patients and their families. Clinical trials for children and adolescents diagnosed with cancer are generally designed to compare potentially better therapy with current standard therapy. Other types of clinical trials test novel therapies when there is no standard therapy for a cancer diagnosis. Most of the progress in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI website.

Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[3,4,5] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see Late Effects of Treatment for Childhood Cancer.

Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[6] The U.S. Rare Diseases Act of 2002 defines a rare disease as one that affects populations smaller than 200,000 people in the United States. Therefore, all pediatric cancers are considered rare.

The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[7,8] In children and adolescents, the designation of a rare tumor is not uniform among international groups, as follows:

• A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than two cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than two cases per 1 million people were also included in the very rare group due to a lack of knowledge and expertise in the management of these tumors.[9]
• The Children's Oncology Group defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancers, melanomas and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinomas, nasopharyngeal carcinomas, and most adult-type carcinomas such as breast cancers and colorectal cancers).[10] These diagnoses account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years.[4]

  Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers diagnosed in children aged 0 to 14 years and 9.3% of the cancers diagnosed in adolescents aged 15 to 19 years.

These rare cancers are extremely challenging to study because of the relatively few patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the low number of clinical trials for adolescents with rare cancers.

Information about these tumors may also be found in sources relevant to adults with cancer, such as Pancreatic Cancer Treatment and Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment.

References:

1. Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010.
2. American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. Also available online. Last accessed August 23, 2024.
3. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.
4. National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed August 23, 2024.
5. Surveillance Research Program, National Cancer Institute: SEER*Explorer: An interactive website for SEER cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed March 6, 2024.
6. Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr.
7. Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017.
8. DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017.
9. Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019.
10. Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010.

Solid Pseudopapillary Tumor of the Pancreas

Incidence

Solid pseudopapillary tumor of the pancreas, also known as Frantz tumor, is the most common pediatric pancreatic tumor, accounting for up to 70% of cases in most institutional series.[1,2] This tumor has low malignant potential and most commonly affects females of reproductive age (median age, 21 years), Black people, and East Asian people.[2,3,4] There is no known genetic or hormonal factor to explain the strong female predilection, although it has been noted that all tumors express progesterone receptors.[5]

Histology and Genomic Alterations

Histologically, solid pseudopapillary tumor of the pancreas is characterized by a combination of solid, pseudopapillary, and cystic changes. The fragility of the vascular supply leads to secondary degenerative changes and cystic areas of hemorrhage and necrosis.[2,3,4] The cells surrounding the hyalinized fibrovascular stalks form the pseudopapillae.[3]

A highly specific, paranuclear, dot-like immunoreactivity pattern for CD99 has been described.[6]

Variants in the CTNNB1 gene have been identified in more than 90% of these tumors.[7]

Diagnosis

Solid pseudopapillary tumor of the pancreas is a very friable tumor, and tumor rupture and hemoperitoneum have been reported.[2,3,4] Tumors can occur throughout the pancreas and are often exophytic. On imaging, the mass shows typical cystic and solid components, with intratumoral hemorrhage and a fibrous capsule.[3]

A retrospective review of the National Cancer Database identified 21 pediatric patients (younger than 18 years) and 348 adult patients with solid pseudopapillary neoplasm of the pancreas.[8] When compared with their adult counterparts, the children had similar disease severity at presentation, received similar treatments, and experienced equivalent postoperative outcomes.

Treatment of Solid Pseudopapillary Tumor of the Pancreas

The outcomes of patients with solid pseudopapillary tumors of the pancreas are excellent, with 10-year survival rates exceeding 95%.[5]

Treatment options for solid pseudopapillary tumor of the pancreas include the following:

1. Surgery.
2. Chemotherapy.

Surgery

Treatment of solid pseudopapillary tumor of the pancreas is primarily surgical. However, preoperative and operative spillage is not unusual.[9] Whipple procedures (pancreaticoduodenectomy) are often necessary, but non-Whipple, pancreatic-sparing resections may be possible with a pancreatico-jejunostomy procedure. Surgery is usually curative, although local recurrences occur in 5% to 15% of cases.[4]

A retrospective review of the Italian Pediatric Rare Tumor Registry identified 43 pediatric patients diagnosed with solid pseudopapillary tumor of the pancreas between 2000 and 2018.[10][Level of evidence C1] The median age at diagnosis was 13.2 years (range, 7–18 years). Only one patient presented with metastatic disease.

• At follow-up (median, 8.4 years; range, 0–17 years), one recurrence occurred in a patient who had intraoperative rupture, and all patients were alive.

A study identified 38 children aged 0 to 18 years who were diagnosed with pseudopapillary solid tumor of the pancreas between 2008 and 2022 in a German registry.[11] Patients were a median age of 14.5 years at diagnosis (range, 8–18 years), with a female preponderance (81.6%). The pancreatic tail was the most frequent location of the tumor. All patients underwent surgical resection.

• No recurrences occurred during follow-up, although two patients underwent resection with microscopic residual disease.

Chemotherapy

Single-agent gemcitabine is reportedly effective in cases of unresectable or metastatic disease.[12] Metastatic disease, usually in the liver, may occur in up to 15% of cases.[2,3,4,5,6]

References:

1. Rojas Y, Warneke CL, Dhamne CA, et al.: Primary malignant pancreatic neoplasms in children and adolescents: a 20 year experience. J Pediatr Surg 47 (12): 2199-204, 2012.
2. Dall'igna P, Cecchetto G, Bisogno G, et al.: Pancreatic tumors in children and adolescents: the Italian TREP project experience. Pediatr Blood Cancer 54 (5): 675-80, 2010.
3. Chung EM, Travis MD, Conran RM: Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 26 (4): 1211-38, 2006 Jul-Aug.
4. Papavramidis T, Papavramidis S: Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg 200 (6): 965-72, 2005.
5. Estrella JS, Li L, Rashid A, et al.: Solid pseudopapillary neoplasm of the pancreas: clinicopathologic and survival analyses of 64 cases from a single institution. Am J Surg Pathol 38 (2): 147-57, 2014.
6. Laje P, Bhatti TR, Adzick NS: Solid pseudopapillary neoplasm of the pancreas in children: a 15-year experience and the identification of a unique immunohistochemical marker. J Pediatr Surg 48 (10): 2054-60, 2013.
7. Rodriguez-Matta E, Hemmerich A, Starr J, et al.: Molecular genetic changes in solid pseudopapillary neoplasms (SPN) of the pancreas. Acta Oncol 59 (9): 1024-1027, 2020.
8. Leraas HJ, Kim J, Sun Z, et al.: Solid Pseudopapillary Neoplasm of the Pancreas in Children and Adults: A National Study of 369 Patients. J Pediatr Hematol Oncol 40 (4): e233-e236, 2018.
9. Vasudevan SA, Ha TN, Zhu H, et al.: Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study. Pediatr Blood Cancer 67 (9): e28425, 2020.
10. Crocoli A, Grimaldi C, Virgone C, et al.: Outcome after surgery for solid pseudopapillary pancreatic tumors in children: Report from the TREP project-Italian Rare Tumors Study Group. Pediatr Blood Cancer 66 (3): e27519, 2019.
11. Jentzsch C, Fuchs J, Agaimy A, et al.: Solid pseudopapillary neoplasms of the pancreas in childhood and adolescence-an analysis of the German Registry for Rare Pediatric Tumors (STEP). Eur J Pediatr 182 (12): 5341-5352, 2023.
12. Maffuz A, Bustamante Fde T, Silva JA, et al.: Preoperative gemcitabine for unresectable, solid pseudopapillary tumour of the pancreas. Lancet Oncol 6 (3): 185-6, 2005.

Pancreatoblastoma

Incidence and Risk Factors

Pancreatoblastoma accounts for 10% to 20% of all pancreatic tumors during childhood. It is the most common pancreatic tumor of young children and typically presents in the first decade of life, with a median age at diagnosis of 5 years.[1,2]

Patients with Beckwith-Wiedemann syndrome have an increased risk of developing pancreatoblastoma. This syndrome is identified in up to 60% of children who develop pancreatoblastoma during early infancy and in 5% of children who develop pancreatoblastoma later in life.[3] Pancreatoblastoma has also been associated with familial adenomatous polyposis syndromes.[4]

Clinical Presentation

Although approximately one-half of pancreatoblastoma cases originate in the head of the pancreas, jaundice is uncommon. Close to 80% of the tumors secrete alpha-fetoprotein, which can be used to measure response to therapy and monitor for recurrence.[2] In some cases, the tumor may secrete adrenocorticotropic hormone or antidiuretic hormone, and patients may present with Cushing syndrome and the syndrome of inappropriate antidiuretic hormone secretion.[3] Metastases are present in 30% to 40% of patients, usually involving liver, lungs, and lymph nodes.[2]

Histology and Molecular Features

Pancreatoblastoma is thought to arise from the persistence of the fetal analogue of pancreatic acinar cells. Pathological examination shows an epithelial neoplasm with an arrangement of acinar, trabecular, or solid formations separated by dense stromal bands.[1] These tumors will often have activation of WNT signaling (most commonly caused by somatic variants in CTNNB1). IGF2 gene alterations have also been frequently observed in individuals with pancreatoblastoma. These findings suggest that pancreatoblastoma might result from the disruption of normal pancreas differentiation.[5,6]

Treatment of Pancreatoblastoma

The European Cooperative Study Group for Pediatric Rare Tumors within the PARTNER project (Paediatric Rare Tumours Network - European Registry) has published consensus guidelines for the diagnosis and treatment of childhood pancreatoblastoma.[7]

Using a multimodality approach, close to 80% of patients can be cured.[2]

Treatment options for pancreatoblastoma include the following:

1. Surgery.
2. Chemotherapy.
3. Other treatments.

Surgery

Surgery is the main treatment for pancreatoblastoma, and a complete surgical resection is required for cure. Because of the common origin of the tumor in the head of the pancreas, a Whipple procedure is usually required.[8,9]

Chemotherapy

For large, unresectable, or metastatic tumors, preoperative chemotherapy is indicated. Pancreatoblastoma often responds to chemotherapy. A cisplatin-based regimen is usually recommended. The PLADO regimen, which includes cisplatin and doxorubicin, is most commonly used. Treatment is modeled after the management of hepatoblastoma, with two to three cycles of preoperative therapy, followed by resection and adjuvant chemotherapy.[2,4,10,11]

Patients with relapsed or persistent pancreatoblastoma have responded to treatment with gemcitabine (one case) [12] and vinorelbine and oral cyclophosphamide (two cases).[13]

Other treatments

Although radiation therapy has been used for unresectable tumors and relapsed cases, its role in the treatment of microscopic disease after surgery has not been defined.[4]

High-dose chemotherapy with autologous hematopoietic stem cell rescue has been reported to be effective in selected cases.[10,14]

References:

1. Chung EM, Travis MD, Conran RM: Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 26 (4): 1211-38, 2006 Jul-Aug.
2. Bien E, Godzinski J, Dall'igna P, et al.: Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). Eur J Cancer 47 (15): 2347-52, 2011.
3. Chisholm KM, Hsu CH, Kim MJ, et al.: Congenital pancreatoblastoma: report of an atypical case and review of the literature. J Pediatr Hematol Oncol 34 (4): 310-5, 2012.
4. Glick RD, Pashankar FD, Pappo A, et al.: Management of pancreatoblastoma in children and young adults. J Pediatr Hematol Oncol 34 (Suppl 2): S47-50, 2012.
5. Honda S, Okada T, Miyagi H, et al.: Spontaneous rupture of an advanced pancreatoblastoma: aberrant RASSF1A methylation and CTNNB1 mutation as molecular genetic markers. J Pediatr Surg 48 (4): e29-32, 2013.
6. Isobe T, Seki M, Yoshida K, et al.: Integrated Molecular Characterization of the Lethal Pediatric Cancer Pancreatoblastoma. Cancer Res 78 (4): 865-876, 2018.
7. Bien E, Roganovic J, Krawczyk MA, et al.: Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations. Pediatr Blood Cancer 68 (Suppl 4): e29112, 2021.
8. Vasudevan SA, Ha TN, Zhu H, et al.: Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study. Pediatr Blood Cancer 67 (9): e28425, 2020.
9. Lindholm EB, Alkattan AK, Abramson SJ, et al.: Pancreaticoduodenectomy for pediatric and adolescent pancreatic malignancy: A single-center retrospective analysis. J Pediatr Surg 52 (2): 299-303, 2017.
10. Dall'igna P, Cecchetto G, Bisogno G, et al.: Pancreatic tumors in children and adolescents: the Italian TREP project experience. Pediatr Blood Cancer 54 (5): 675-80, 2010.
11. Défachelles AS, Martin De Lassalle E, Boutard P, et al.: Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients. Med Pediatr Oncol 37 (1): 47-52, 2001.
12. Belletrutti MJ, Bigam D, Bhargava R, et al.: Use of gemcitabine with multi-stage surgical resection as successful second-line treatment of metastatic pancreatoblastoma. J Pediatr Hematol Oncol 35 (1): e7-10, 2013.
13. Dhamne C, Herzog CE: Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. J Pediatr Hematol Oncol 37 (6): e378-80, 2015.
14. Hamidieh AA, Jalili M, Khojasteh O, et al.: Autologous stem cell transplantation as treatment modality in a patient with relapsed pancreatoblastoma. Pediatr Blood Cancer 55 (3): 573-6, 2010.

Islet Cell Tumors

Incidence

Islet cell tumors represent approximately 15% of pediatric pancreatic tumors in most series.[1,2,3] These tumors usually present in middle age and may be associated with multiple endocrine neoplasia type 1 (MEN1) syndrome. Less than 5% of islet cell tumors occur in children.[4] For more information, see Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment.

A single referral center in Russia retrospectively identified 22 children (aged 5–16 years) who were diagnosed with insulinomas.[5] Five patients (23%) had multiple pancreatic lesions. All children underwent surgical treatment. Two patients were diagnosed with metastatic insulinomas. One of them had metastases at the time of insulinoma diagnosis, while the other was diagnosed with liver metastases 8 years after surgery. Eight children (36%) were found to carry MEN1 variants (inherited, n = 5; de novo, n = 1; no data, n = 2). Children with MEN1 syndrome had a significantly higher number of pancreatic tumors than children without MEN1 syndrome. All of the MEN1 carriers developed additional MEN1 symptoms during the following 2 to 13 years. After five patients were diagnosed with inherited MEN1 syndrome, previously undiscovered MEN1 manifestations were found in seven family members.

Clinical Presentation, Risk Factors, and Diagnosis

The most common type of functioning islet cell tumor is insulinoma, followed by gastrinoma.

• Insulinoma. Patients with insulinoma present with fasting hyperinsulinemic hypoglycemia. In young children, presentation may include behavioral problems, seizures, or coma.
• Gastrinoma. Gastrinoma presents with Zollinger-Ellison syndrome, with recurrent peptic ulcers in uncommon locations and diarrhea caused by gastric hypersecretion. While most insulinomas are benign, a significant proportion of gastrinomas are malignant.[3]
• ACTHoma and VIPoma. Other less common tumors seldom seen in children are the ACTHoma, which presents as Cushing syndrome, and the VIPoma, which presents as Verner-Morrison syndrome.

Nonfunctioning tumors are extremely rare in pediatrics, except when associated with MEN1 syndrome. Islet cell tumors are typically solitary. When multiple tumors are present, a diagnosis of MEN1 syndrome should be considered.

On imaging, these tumors are usually small and well defined. Somatostatin receptor scintigraphy is useful for detecting the location of islet cell tumors. However, only 60% to 70% of tumors express somatostatin receptors.[4]

Treatment of Islet Cell Tumors

Treatment options for islet cell tumors include the following:

1. Surgery.
2. Chemotherapy.
3. Mammalian target of rapamycin (mTOR) inhibitor therapy.

Treatment of islet cell tumors includes medical therapy for control of the syndrome and complete surgical resection.[6] For patients with malignant tumors and unresectable or metastatic disease, chemotherapy and mTOR inhibitors are recommended.

The management of these tumors in children follows the consensus guidelines established for adult patients.[3,7] For more information, see Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment.

References:

1. Rojas Y, Warneke CL, Dhamne CA, et al.: Primary malignant pancreatic neoplasms in children and adolescents: a 20 year experience. J Pediatr Surg 47 (12): 2199-204, 2012.
2. Dall'igna P, Cecchetto G, Bisogno G, et al.: Pancreatic tumors in children and adolescents: the Italian TREP project experience. Pediatr Blood Cancer 54 (5): 675-80, 2010.
3. Jensen RT, Cadiot G, Brandi ML, et al.: ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology 95 (2): 98-119, 2012.
4. Chung EM, Travis MD, Conran RM: Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 26 (4): 1211-38, 2006 Jul-Aug.
5. Melikyan M, Gubaeva D, Shadrina A, et al.: Insulinoma in childhood: a retrospective review of 22 patients from one referral centre. Front Endocrinol (Lausanne) 14: 1127173, 2023.
6. Vasudevan SA, Ha TN, Zhu H, et al.: Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study. Pediatr Blood Cancer 67 (9): e28425, 2020.
7. Kulke MH, Benson AB, Bergsland E, et al.: Neuroendocrine tumors. J Natl Compr Canc Netw 10 (6): 724-64, 2012.

Pancreatic Carcinoma

Incidence and Risk Factors

Pancreatic carcinomas are extremely rare in children. These malignancies represent less than 5% of pediatric pancreatic tumors and include the following:[1,2]

• Acinar cell carcinoma. Although rare in pediatrics, acinar cell carcinoma is more common than ductal adenocarcinoma, the most common pancreatic carcinoma in adults. Acinar cell carcinoma is considered to be the adult counterpart of pancreatoblastoma, and histological differentiation between these entities may be difficult.[3]
• Ductal adenocarcinoma. Ductal adenocarcinoma is rare in the first four decades of life and even rarer during childhood and adolescence.[4] Ductal adenocarcinoma is associated with several cancer predisposition syndromes, including hereditary pancreatitis (PRSS1 variants), familial atypical mole and multiple melanoma (CDKN2A variants), Peutz-Jeghers syndrome and other hereditary nonpolyposis colon carcinomas (STK11 and germline mismatch repair genes), and syndromes associated with DNA repair gene variants (such as BRCA2 and ATM).[5]

  For more information, see the following summaries:

  • Genetics of Colorectal Cancer.
  • Genetics of Skin Cancer.

Clinical Presentation

Presenting symptoms are nonspecific and are related to local tumor growth. However, 4% to 15% of adult patients with acinar cell carcinoma may present with a lipase hypersecretion syndrome, manifesting as peripheral polyarthropathy and painful subcutaneous nodules.

Treatment of Pancreatic Carcinoma

For information about the treatment of pancreatic carcinoma, see Pancreatic Cancer Treatment.

References:

1. Rojas Y, Warneke CL, Dhamne CA, et al.: Primary malignant pancreatic neoplasms in children and adolescents: a 20 year experience. J Pediatr Surg 47 (12): 2199-204, 2012.
2. Dall'igna P, Cecchetto G, Bisogno G, et al.: Pancreatic tumors in children and adolescents: the Italian TREP project experience. Pediatr Blood Cancer 54 (5): 675-80, 2010.
3. Chung EM, Travis MD, Conran RM: Pancreatic tumors in children: radiologic-pathologic correlation. Radiographics 26 (4): 1211-38, 2006 Jul-Aug.
4. Lüttges J, Stigge C, Pacena M, et al.: Rare ductal adenocarcinoma of the pancreas in patients younger than age 40 years. Cancer 100 (1): 173-82, 2004.
5. Rustgi AK: Familial pancreatic cancer: genetic advances. Genes Dev 28 (1): 1-7, 2014.

Treatment Options Under Clinical Evaluation for Childhood Pancreatic Cancer

Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, see the ClinicalTrials.gov website.

Latest Updates to This Summary (08 / 13 / 2024)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

This summary was comprehensively reviewed.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric pancreatic cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

• be discussed at a meeting,
• be cited with text, or
• replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Childhood Pancreatic Cancer Treatment are:

• Denise Adams, MD (Children's Hospital Boston)
• Karen J. Marcus, MD, FACR (Dana-Farber Cancer Institute/Boston Children's Hospital)
• William H. Meyer, MD
• Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)
• Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)
• Alberto S. Pappo, MD (St. Jude Children's Research Hospital)
• D. Williams Parsons, MD, PhD (Texas Children's Hospital)
• Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)
• Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)
• Stephen J. Shochat, MD (St. Jude Children's Research Hospital)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

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Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

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The preferred citation for this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Pancreatic Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/pancreatic/hp/child-pancreatic-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 31661209]

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Last Revised: 2024-08-13